Tourette Syndrome

This article provides a comprehensive overview of Tourette syndrome, focusing on its symptoms, causes, and treatment approaches, along with supportive resources for parents and children.

Key Takeaways:

• Tourette syndrome is often misunderstood by the public. It is a neurological condition characterized by involuntary movements and/or sounds, called tics.

• Symptoms typically emerge in childhood and often lessen significantly by adolescence and adulthood, although some individuals may continue to be affected.

• Cognitive behavioral interventions and lifestyle strategies can help manage symptoms, although there is currently no known cure. Along with management, spreading awareness and education is essential.

Understanding Tourette Syndrome

Tourette syndrome is a neurodevelopmental disorder characterized by sudden, uncontrollable movements and/or sounds called tics. In the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), it is classified as a tic disorder, which is a group of specific neurodevelopmental disorders within the motor disorders category. Other tic disorders include persistent motor or vocal tic disorder and provisional tic disorder.

Symptoms of Tourette syndrome typically begin between ages 5 and 10, particularly as motor tics in the head and neck area, although symptom onset can occur earlier or later in some cases. In general, motor tics (e.g. head jerking, blinking) occur before vocal tics (e.g. sniffing, grunting).^[1][2]

The tics within Tourette syndrome vary greatly from person to person in terms of severity, frequency, and general presentation. For example, some individuals may only experience mild tics that occur relatively infrequently and don’t cause much disruption, while more severe cases may involve frequent, extreme tics that cause dysfunction and even disability.^[1]

How common is It?

Tourette syndrome (TS) affects up to 1% of the general population. In children, estimates suggest that it affects between 3 and 8 out of every 1,000 children.^[3]

However, tics often go unrecognized, and up to 50% of children may go undiagnosed. So, the exact prevalence isn’t definitively known.^[1]

Who does it affect?

Tourette syndrome is three to four times more common in boys than in girls. It is also more common in children than adults, in large part because most children with this disorder experience partial or complete resolution of symptoms by adulthood.^[1]

Symptoms

The primary symptoms of Tourette syndrome are tics, which are grouped into motor tics and vocal/phonic tics. Specific examples of these symptoms are listed below:^[1][2]

Motor Tics

• Eye blinking

• Facial grimacing

• Head bobbing/jerking

• Neck jerking

• Shoulder shrugging

• Neck stretching

• Arm jerking

Individuals with Tourette syndrome may also perform complex motor tics, which can include a combination of the above tics together, or different movements altogether, such as hopping, jumping, bending, or twirling.^[1][2]

Vocal/Phonic Tics

• Throat clearing

• Grunting

• Sniffing

• Hooting

• Shouting

• Barking

In addition to these simple vocal tics, individuals with this disorder may also have complex vocal tics. This includes repeating one’s own words or phrases, repeating others’ words or phrases (echolalia), or using vulgar language/swear words (coprolalia).^[1][2]

It’s important to note that these complex vocal tics are often the stereotypical symptoms portrayed in the media, but these are relatively rare.

Individual Differences

Tics can vary greatly from person to person in terms of the exact type, frequency, and severity. They can range from mild and manageable to severe and disruptive, potentially interfering with interpersonal relationships and daily functioning.

Also, some people are able to suppress or manage their tics to some degree. However, this usually leads to a significant build-up of tension where they feel the tic has to be performed. Tics may also become more intense during periods of excitement, anxiety, fatigue, or stress. However, symptom changes can also occur without a clear trigger.^[1][2]

Social Difficulties of Having TS

Tourette syndrome often leads to various social difficulties, including stigmatization and difficulty forming or maintaining relationships. Due to limited public awareness, individuals with TS are sometimes unfairly labeled or misunderstood, leading to social stigma.

These challenges are especially common in school settings, where peer judgment and social dynamics can cause significant anxiety and distress for individuals with TS, leading to social isolation and withdrawal.

What Causes TS?

The exact causes of TS are not fully understood. However, it has been linked to genetic factors and abnormalities in certain brain regions, circuits, and neurotransmitters, including dopamine, serotonin and GABA.^[2][3]

Other possible causes include immune system dysfunction, brain inflammation, and certain pre- or perinatal events, such as maternal smoking, preterm birth, or perinatal injury.

Can it be Prevented?

Tourette syndrome cannot be prevented, as it is a neurological disorder with no clearly identifiable cause.

Risk Factors

There are several risk factors for TS, including genetics. 52% of children with TS have a family history of the disorder and are up to ten times more likely than the general population to have a first-degree relative with TS.^[3]

Another risk factor is biological sex. Males are three to four times more likely than females to have TS. In addition, males with a genetic predisposition are more likely to develop tics, whereas at-risk females are more likely to develop obsessive-compulsive symptoms.^[2]

Related Conditions of TS

An estimated 83% of people with TS have co-occurring mental health disorders and/or other medical conditions. Some of the most common conditions that co-occur with TS are:^[1][2][3]

• Attention-deficit hyperactivity disorder (ADHD)

• Obsessive-compulsive disorder (OCD)

• Behavioral or conduct issues

• Includes aggression, rage, social defiance

• May also include a more severe form called oppositional-defiant disorder (ODD)

• Anxiety, whether sub-clinical or an anxiety disorder

• Learning disability

• Deficits in social skills/social functioning

• Touble developing social skills, forming and maintaining social relationships with peers, family members, others

• Sensory processing issues

• Strong sensory preferences

• Sensitivities related to touch, sound, taste, smells, and movement

• Trouble falling or staying asleep

Diagnosing TS

Diagnosing Tourette syndrome primarily involves a comprehensive history and physical examination conducted by an experienced provider. Primary care clinicians, physicians, and mental healthcare specialists can all diagnose TS, provided they have relevant training and experience.

During the physical examination, the clinician looks for the presence of motor or vocal tics, which is assessed in conjunction with the patient’s clinical history. The most widely accepted scale for measuring TS is the Yale Global Tic Severity Scale. This scale evaluates specific tic symptoms based on their number, frequency, severity, intensity, complexity, and overall impact on functioning.^[3]

Blood, lab, or imaging tests are rarely necessary, although they may be used in some cases to rule out other possible causes. The clinician will also evaluate for possible comorbid conditions, such as anxiety, OCD, or ADHD.

The specific diagnostic criteria for TS in theDSM-5are:^[3]

• The presence of multiple motor tics and one or more vocal tics at some point during the illness, although not necessarily at the same time

• The tics may change in frequency, but have lasted for more than 1 year after initial onset

• The onset occurred before age 18

• The symptoms are not caused by the use of a substance or a medical condition

Can You be Diagnosed as an Adult?

Yes, you can receive a TS diagnosis as an adult, provided the symptoms started before the age of 18\. In some cases, mild symptoms in childhood may go unnoticed or be misinterpreted, which may lead to a diagnosis never being made. As adults, individuals may become more aware of their symptoms or seek evaluation due to increased awareness about TS.

If you suspect you may have TS, consider discussing your concerns with your healthcare provider for appropriate evaluation and guidance.

Treatment Approaches

There is currently no known cure for Tourette syndrome, although several effective treatment options can help manage symptoms and improve daily functioning. Common TS treatments include medication, behavioral therapies, and psychotherapy.

Behavior Therapy

Behavior therapy is often recommended as the first-line treatment for TS, particularly in children, given the potential side effects of medications. The most commonly used behavioral therapy for TS is Cognitive Behavioral Intervention for Tics (CBIT), which consists of three main components:^[3][4]

• Training people to become more aware of their tics and the urge to tic

• Teaching individuals competing behaviors to use when they feel the urge to tic

• Making changes in daily life that may help reduce tics (e.g, relaxation techniques for stress and anxiety)

Medication

Several medications may be prescribed to help manage TS symptoms. The most commonly used include:^[2][3]

• Antipsychotics (e.g., haloperidol, pimozide, risperidone, aripiprazole)

• Alpha-2-adrenergic agonists (e.g., clonidine, guanfacine)

Possible side effects of antipsychotics include sedation, weight gain, nausea, and dystonia. Possible side effects of alpha-2-adrenergic agonists include sedation, low blood pressure, irritability, and slow heart rate.^[3]

Certain medications may also be prescribed to treat comorbid conditions, such as antidepressants for depression or anxiety, or stimulants for ADHD.^[2][3]

Other Possible Treatments

In cases of severe TS symptoms or when standard treatments are ineffective, other medical options may be considered. This includes botulinum toxin injection and deep brain stimulation.

Botulinum toxin injections are primarily used for severe motor tics that are painful or disabling, though they can be used for other tics too. They work by weakening the nervous system stimulation of muscles, which helps them to relax and reduces spasms and tics.^[3][5]

Deep brain stimulation involves surgically implanting electrodes in specific regions of the brain. These electrodes deliver electrical impulses that can help regulate abnormal brain activity. However, it is generally only considered after all other treatment options are exhausted.^[5]

Managing Tourette Syndrome

Tourette syndrome can cause many challenges, but there are several things individuals can do to help better manage the condition. These include:^[6]

• Engage in sports, exercise, and hobbies (this may help reduce tics and redirect physical and mental energy)

• Partake in creative activities, such as writing, painting, or making music

• Learn as much as you can about TS (ask your doctor any questions you have)

• Practice relaxation strategies, such as deep breathing, meditation, and yoga

• Communicate openly to others about your TS, especially family, friends, and teachers

• Consider joining a TS-centered support group

• Don’t try to suppress tics

• Lean on family and friends for social support

Supporting a Child with Tourette Syndrome

Parents, caregivers, and teachers also have important roles when it comes to supporting a child with Tourette syndrome. Ways to be more supportive of a child with Tourette syndrome include:

• Educate yourself

• This promotes more appropriate and effective responses

• In general, don’t draw attention or react noticeably to tics

• Encourage participation in sports and other activities

• Implement appropriate accommodations for children at school

• E.g. tutoring, smaller/special classes, private study areas

• Communicate with other parents, teachers, and new acquaintances, especially those who don’t fully understand TS

Final Thoughts and Additional Support

Life with Tourette syndrome often poses significant challenges not only due to its symptoms, but also because of the frequent co-occurrence of other conditions. Moreover, the general public’s lack of understanding about this disorder can result in stigmatization and other negative behaviors.

Although there is no cure for TS, many individuals with the disorder can lead fulfilling lives by combining professional treatments with strong social support and lifestyle strategies. If these are implemented, children with TS can enjoy daily life just like any other child, even despite the greater challenges.

Parents and children can also find additional support resources at the following pages:

• Tourette Association of America

• Tourettes Action (UK)

• Center for Disease Control and Prevention: Free materials about Tourette syndrome

• International resources: Here you can find contacts for your specific country who are willing to provide information and support to families.